27th June 2023

Chronic inflammatory demyelinating polyneuropathy

Chronic inflammatory demyelinating polyneuropathy (CIDP) is a subset of rare, immune-mediated polyradiculoneuropathies. Although multiple forms exist, CIDP typically presents with a slowly progressive symmetrical motor neuropathy affecting both proximal and distal peripheral nerves. Sensory impairment may also be identified, although usually less severe than the limb weakness. Examination findings include areflexia with signs of lower motor neuron disease and patchy sensory loss. High protein levels are found on cerebrospinal fluid analysis. Nerve conduction studies are key to diagnosis and will reveal multiple partial conduction blocks and prolonged distal motor latency, suggestive of demyelination. The presence of ‘onion bulb formations’ of axons are a pathognomonic finding frequently seen in biopsy results. CIDP can be treated with intravenous immunoglobulins or plasma exchange, although intravenous high-dose steroids are an alternative.